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Year : 2022  |  Volume : 5  |  Issue : 1  |  Page : 34-40

Isolated pulmonary hypertension without interstitial lung disease with progressive dyspnea in young female needs connective tissue diseases workup: A case report with review of the literature

Department of Pulmonary Medicine, MIMSR Medical College, Latur, Maharashtra, India

Correspondence Address:
Shital Patil
Department of Pulmonary Medicine, MIMSR Medical College, Latur, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/japt.japt_11_22

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Obstructive airway disease is the leading cause of breathlessness in young patients and in the absence of atopy or chest radiograph abnormalities such as nontubular heart and enlarged pulmonary artery mandates more workup. Pulmonary manifestations of mixed connective tissue disease (MCTD) range from bronchiolitis and bronchiectasis to interstitial lung disease and pulmonary hypertension. In this case report, a 25 year old female, presented with progressive dyspnoea and received treatment in line of obstructive airway disease with inhaled bronchodilators with inhaled corticosteroids with clinical worsening and no treatment response even after 24 months with good compliance. Radiological workup documented cardiomegaly with enlarged pulmonary artery in chest X-ray, multidetector computed tomography (MDCT) thorax documented grossly enlarged pulmonary arteries with dilated right heart chambers, and echocardiography documented severe pulmonary hypertension with dilated right atrium and right ventricle. Vasculitis and connective tissue diseases (CTD) workup was documented as strongly positive antinuclear antibody (ANA) with very highly raised titers, with other parameters in ANA blot documented positive antigens such as U1-small nuclear ribonucleoprotein particle, SSA/RO, single-strand DNA, and Scl-70. We have started on tadalafil, ambrisentan, and diuretics, and documented clinical response with increased work performance with improved quality of life with stabilization of pulmonary artery pressures on echocardiography at 12 weeks of treatment. We recommend young female genders with progressive dyspnea with nontubular heart on chest radiograph with or without enlarged pulmonary artery needs prompt workup such as high-resolution computed tomography/MDCT thorax, echocardiography, and ANA blot for early pickup of underlying CTD/MCTD to have successful treatment outcome.

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